Cases reported "Pituitary Diseases"

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1/20. Idiopathic giant-cell granulomatous hypophysitis mimicking acute meningitis.

    A 32-year-old woman presented with severe headache, photophobia, fever, nausea, vomiting, and worsening vision. She had also noted several months of amenorrhea. She was febrile to 38.9 degrees C. Laboratory evaluation revealed a markedly elevated erythrocyte sedimentation rate. Lumbar puncture revealed a cerebrospinal fluid lymphocytic pleocytosis and an elevated protein level. Endocrine studies revealed evidence of panhypopituitarism without diabetes insipidus. A magnetic resonance imaging study showed a 2-cm pituitary mass with optic chiasmal compression. The patient had a trans-sphenoidal resection of the mass. pathology revealed multinucleated giant cells in necrotic debris, but no evidence of pituitary tumor. Studies looking for evidence of systemic granulomatous disease were negative. The patient was considered to have idiopathic giant-cell granulomatous hypophysitis. After surgery, the patient's vision improved and hormone replacement therapy was initiated. This case illustrates that idiopathic giant-cell granulomatous hypophysitis should be considered in the differential diagnosis of a patient presenting with a pituitary mass, hypopituitarism, and meningitis-like symptoms.
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2/20. Idiopathic giant cell granulomatous hypophysitis with hypopituitarism, right abducens nerve paresis and masked diabetes insipidus.

    A 38-year-old man presented with headache, fever, and double vision associated with right abducens nerve paresis. He had neither nuchal rigidity nor visual field defect. Laboratory data revealed elevated erythrocyte sedimentation rate (ESR), eosinophilia, and lymphocytic pleocytosis in the cerebrospinal fluid (CSF). Provocation tests of pituitary hormones showed partial hypopituitarism. magnetic resonance imaging (MRI) revealed swelling of the hypophysis and a mass lesion expanding into the right cavernous sinus. The supplement dose of dexamethasone for hypothalamic hypocortisolism manifested diabetes insipidus. biopsy, carried out through the transsphenoidal approach, revealed giant cell granuloma. Systemic granulomatous diseases were ruled out, and the lesion was considered to be idiopathic giant cell granulomatous hypophysitis. Right abducens nerve paresis, diabetes insipidus and dysfunction of the anterior lobe were amended by the treatment with prednisolone for 4 months, and findings of the pituitary gland and stalk were normalized. The present case shows that glucocorticoid has an effect on amendment of idiopathic giant cell granulomatous hypophysitis.
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3/20. Natural course of lymphocytic infundibuloneurohypophysitis.

    Natural course of lymphocytic infundibuloneurohypophysitis is poorly understood. A 49-year-old male had noticed being unnaturally thirsty since about two years previously. An enlargement of the pituitary stalk and pituitary gland was thus observed by MR at that time. However, no medical care had been given. Two years later, he was admitted to our hospital due to headache in addition to panhypopituitarism. The histologic features included T cell dominant lymphocytes infiltrating prominently the entire pituitary gland with a small amount of multinucleated giant cells, focal and small necrosis, cholesterin crystals and granuloma. Neither tuberculosis nor Langerhans histiocytosis were observed. In addition, the patient was found to have a unique massive well-encapusulated lesion in the sphenoid sinus, just below the pituitary fossa, consisting of serous fluid, normal columnar epithelium and submucosal fibrosis. This patient had a fairly typical clinical manifestation of lymphocytic infundibuloneurohypophysitis with invason of the posterior lobe and the stalk. The lesion became chronic and leaked to the sphenoid sinus. As a result, chronic hypophysitis with granuloma formation thus occurred. This case may show the course of this disease if not treated.
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4/20. Xanthogranuloma of the Erdheim-Chester type within the sellar region: case report.

    Manifestations of erdheim-chester disease in the central nervous system are very rare. Cases with localization in the retroorbital space, hypothalamic area and posterior pituitary as well as intracerebral lesions are known. In our neurosurgical unit, a 51-year-old male patient with a history of hypophyseal insufficiency and visual deficits underwent surgery for a pituitary lesion. Histological and immunohistochemical examination revealed a xanthogranulomatous lesion composed of very large CD68-positive foam cells with small nuclei and some Touton-like giant cells, histiocytes, as well as loci with small lymphocytes and isolated eosinophilic granuolcytes, embedded in fibrotic tissue. Based on these findings, the histological diagnosis was a xanthogranuloma of the Erdheim-Chester type.
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5/20. Xanthomatous pituitary lesions: a report of two cases and review of the literature.

    We describe two young men with cystic pituitary enlargement on magnetic resonance imaging (MRI) causing hypopituitarism. The first patient presented acutely unwell with headache and vomiting associated with anterior and posterior pituitary dysfunction. The second patient presented with hypopituitarism after a long history of hypogonadism. In both cases yellow/brown fluid was found at surgery and histological examination revealed inflammatory infiltrate with foamy histiocytes, lymphocytes and multinucleated giant cells containing cholesterol clefts. Full recovery of pituitary function occurred after surgery in the first but not the second patient. The first case is the first documented case of xanthomatous hypophysitis with recovery of pituitary function following surgery. The cases differed in duration of disease, as indicated by the long history of symptoms, the histological finding of marked fibrosis and the lack of recovery of pituitary function in the second. Xanthomatous pituitary lesions categorized in the literature as xanthomatous hypophysitis, xanthogranulomatous hypophysitis and xanthogranuloma of the sellar region have overlapping histological features. Our two cases revealed histological features that do not fit completely into any of the categories but share features of all three. These findings suggest that the various xanthomatous lesions of the sellar region may be a spectrum of a common inflammatory process rather than distinct pathological entities.
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6/20. Xanthogranulomatous hypophysitis mimicking a pituitary neoplasm.

    Hypophysitis is an inflammatory disease of the pituitary gland that clinically and radiologically mimics pituitary tumors. We report here a case of xanthogranulomatous hypophysitis mimicking a pituitary neoplasm.A 65-yr-old woman presented with weight loss, fatigue, and visual disturbance. Computed tomography demonstrated a round cystic low-density mass with calcification in the sella. A T1-weighted magnetic resonance imaging scan showed most of the mass as hyperintense. The capsule of the mass was strongly enhanced by gadolinium. Endocrinologic examination revealed hypocorticism and hypothyroidism. diabetes insipidus (DI) developed after the administration of hydrocortisone. The patient also had hallucination and delusions of persecution. Transsphenoidal surgery was performed. Histologic examination of the removed tissue showed central necrosis surrounded by accumulation of foamy cells and epithelioid cells. Several multinucleated giant cells were also seen. The foamy cells and epithelioid cells were immunopositive for Kp-1, a marker of macrophages. The patient made an uneventful postoperative recovery. Although DI and hypofunction of adenohypophysis persisted, the visual disturbance and psychiatric disorder were resolved. We have described an unusual inflammatory lesion of the pituitary in the sellar region that was mimicking neoplasm. A high level of clinical suspicion of inflammatory disorders is necessary for correct diagnosis and optimal management.
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7/20. Sellar granulomatous mass in a pregnant woman with active Crohn's disease.

    At the end of her third pregnancy, a woman with a history of chronic active proctocolitis which had been diagnosed as Crohn's disease complained of deterioration of visual acuity. The patient was found to have hypopituitarism and progressive bitemporal hemianopia caused by an intrasellar mass with suprasellar extension. At transsphenoidal surgery an intrasellar granuloma was found without remnants of pituitary tissue. This sellar granuloma could have been an extra-intestinal granuloma of Crohn's disease. However, the possibility that giant cell granulomatous hypophysitis had been present was considered more likely. The occurrence of this disorder in the postpartum period has not been reported before.
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8/20. Ischemic chiasmal syndrome.

    Twenty-two patients with ischemic chiasmal syndrome (ICS) have been examined by means of magnification angiography and polytomographic encephalography. The causes of ICS may be divided into 5 categories: (1) mechanical compression of the chiasm by ectatic redundant anterior cerebral arteries, (2) atherosclerotic vascular occlusions, (3) optochiasmal arachnoidal fibrosis, (4) various forms of arteritis especially giant cell (cranial) arteritis, and (5) post-partum pituitary necrosis associated with ICS. The most common cause of ICS appears to be optochiasmal arachnoiditis which occasionally developed some time after a craniotomy and radiation therapy for pituitary or other juxta-sellar tumors; similar fibrotic change may develop following steroid treatment of granulomatous lesions.
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9/20. Isolated growth hormone deficiency associated with a giant arteriovenous varix.

    A 6.5-year-old girl with short stature (height age, 3 years; bone age, 1.75 years) had isolated growth hormone deficiency. Preoperative computed tomography and magnetic resonance imaging demonstrated a large, well-demarcated, homogeneous mass above the dorsum sellae with a density consistent with flowing blood. Vertebral angiography showed a giant intracranial varix caused by an arteriovenous fistula that originated at the apex of the basilar artery. At operation, an aneurysm clip was placed at the origin of the fistula, and occlusion of the fistula was confirmed by intraoperative digital subtraction angiography. There were no permanent neurological sequelae. Despite shrinkage of the varix, demonstrated by follow-up computed tomography, growth hormone deficiency persisted postoperatively. Biosynthetic growth hormone therapy was initiated 6.5 months after surgery and resulted in a height increment of 8.2 cm after 9 months of treatment.
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10/20. Case report: idiopathic giant cell granuloma of the hypophysis: an unusual cause of panhypopituitarism.

    A 37-year-old man presented with headache and reduced libido. He suffered from panhypopituitarism, and the walls of the pituitary fossa were noted to be thickened, sclerotic, and irregular. Computed tomography scans showed an intrasellar and suprasellar, patchy enhancing tumor. From a biopsy taken at transsphenoidal surgery, a giant cell granuloma was diagnosed, for which there was no obvious cause. Inflammatory disease within the pituitary fossa should be suspected if a reaction is seen in the surrounding bone.
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